• Nalaka de Silva

Ménière's Disease

Updated: Jun 16, 2019


Please note that the following is a general guideline only. For a full assessment, exclusion of any other underlying cause for your symptoms and an individualised treatment approach, you will need to be seen by a qualified specialist.





Definition

A disorder of the inner ear with intermittent episodes of vertigo, tinnitus and fluctuating sensorineural hearing loss



Epidemiology

Incidence: 4 per 100 000

Age: onset 4th decade / uncommon in children

Sex: M=F

Race: More in European decent 1/2500/ rare in Africans

Familial: 20% have positive family Hx





AETIOLOGY

Primary Unknown. Theories are

1 autoimmune

2 subclinical HSV 1 infection

3 ischaemia of parts of the balance organ


Some of the known causes are

1 Known congenital anomalies of the balance organ

2 Trauma

3 Otosclerosis of the inner ear (rare condition)

4 Some know autoimmune conditions

5 Some known viral infections




PATHOPHYSIOLOGY


Understanding the pathophysiology helps understands the symptoms and treatment of this condition.


The ears act as balance organs in space. This is similar to how two jet engines (balance organs) in either side of a plane allows the pilot (your brain) to fly steadily.


Misfiring of an engine makes it impossible for the pilot to fly straight. Similarly, transient misfiring of one balance organ leads to vertigo as it does not allow for the brain to adapt.


However, the complete loss of one jet engine will allow the pilot to fly straight with the existing engine. Similarly, complete damage to the cochlea and balance organ will allow the brain to adapt.


Initially, Meniere's disease causes transient damage or misfiring, leading to vertigo. However, longer term injuries lead burnout of the organ leading to permanent hearing loss through resolving vertigo.



The cause for Menier'rs is an abnormality in the production and flow of the endolymphatic fluid (fluid within the cochlea and the balance organ) This leads to distention of the endolymphatic space (space within the cochlea) causing a sense of ear fullness and tinnitus. When enough endolymph builds up behind the obstruction, there may be sudden outflow across the obstruction toward the sac causing vertigo. Once the pressure is released, symptoms tend to settle, usually over hours.






CLINICAL

vertigo

Usually commences with fullness in the ear.

Acute attacks most commonly last 2-3 hours, unusual to last more than 1 day

Vertigo ceases spontaneously in 60% of patients in 2 years due to damage or burnout of the balance organ.



Hearing loss and ear fullness

Occurs at the commencement of attacks of vertigo. Hearing loss is transient, affecting only the lower frequencies at first and recovers after attacks.

Over time the hearing loss becomes a permanent hearing loss due to damage to the cochlea and balance organ. (this dame causes vertigo the cease)


Tinnitus

Non-pulsatile, whistling or roaring.




Staging of the Disease (depending on the Clinical sy)


Stage 0

Prodromal stage

Intermittent sx of variable duration


Stage 1 (50% of patients)

Intermittent attacks of vertigo dominate

Hearing recovers between attacks

Tinnitus lessens (sometimes disappears) between attacks


Stage 2 (25% of patients)

Intermittent attacks of vertigo

Hearing fluctuates between attacks but does not recover

Tinnitus lessens between attacks

Aural fullness varies


Stage 3 (25% of patients)

Intermittent attacks of vertigo lessen and may cease

Hearing does not fluctuate and remains poor

Tinnitus constant - some people can adapt

Aural fullness may persist





When hearing is still good (hearing preservation treatment)

-Treatment initially aims to reduce pressure

-Low salt low Caffein diet

-A thiazide diuretic works in some (monitor renal function)

-Surgery


When Hearing is not useful-destructive treatment.

Intra tympanic Gentamycin may be given to destroy the labyrinth thereby stop vertigo perception. The patient will rely on the opposite labyrinth for balance. The patients could be selected carefully.








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